Kan taksaa thil thleng bulpui ber chu kan chaw ei hian chakna min pe a ni. Car chhunga gas dah ang mai a ni. Hetah hian kan taksa hian sugar (glucose) atangin chakna a hmu thin. He thil thleng zawng zawng hi hormone
insulin tih hian a thunun a ni . He insulin hian thisen chhunga sugar awm chu chakna mamawh cell-ah thawn turin a hrilh a ni. Cell kawngkhar hawn a, sugar luh tir ang mai a ni a, mahse, Rabson-Mendenhall Syndrome vei taksaah chuan he thil hi a thleng dik lo. Chu chu an taksain insulin a hmang tha thei lo tihna a ni. Hei hi thil awmdan tlemte tak a ni.
Chuti a nih chuan he syndrome hi eng nge ni tak tak?
Insulin hian a hna a thawh that theih loh chuan naupang thanlenna kawngah direct-in a nghawng a ni. Ngaihtuah teh, hei hian a nghawng chu nausen a pian hma, chu chu nu pum chhunga a la awm lai pawhin a intan a ni. Hetiang natna vei naute hi an lian lo tlangpui. Nau piang hnuah pawh an
thau chhoh dan leh an taksa than chhoh dan hi a zawi zawi hle. Damdawi lam hawi chuan Rabson-Mendenhall syndrome hi natna chi khat, pawl lian tak, severe insulin resistance syndromes an tih zinga mi a ni. Donohue syndrome leh type A insulin resistance syndrome te hi natna dang pahnih he pawla tel an ni.
Hetiang dinhmun a lo awm chhan hi eng nge ni?
A awlsam zawngin, hei hi genetic condition a ni. Chu chu
nu leh pa atanga naupang hnena rochun a ni tihna a ni . Hei hi kan taksaa `INSR` an tih gene-a glitch awm vang a ni. Tunah chuan hetiang thil hi engtin nge a lo awm tih i lo en ang u. Kan taksa cell tinah hian gene tin copy pahnih a awm a. Pakhat chu nu hnen atangin, pakhat chu pa hnen atangin. Naupangin Rabson-Mendenhall syndrome a neih theih nan naupangin a dawn `INSR` gene copy pahnih hian a sawi dik lohna hi a nei vek tur a ni. Copy pakhat chauhin a chhiatna a neih chuan natna hi a lo awm lo ang. Phên dangin sawi ila, naupangin he natna a neih theih nân nu leh pa pahnih hian he gene chhia copy khat leh gene hrisel copy khat an neih a ngai a ni. Tichuan, naupang chuan an pahnih hnen atangin copy chhia pahnih chu accident-in a dawng tur a ni. Hei vang hian a tlem em em a, a chhan chu a tlangpuiin vawi li zinga vawi thum a thleng ngai lo.
Hetiang natna lan chhuah dan hi engte nge ni?
Naupang dam
kum khatna atang hian natna lanchhuahna hi a lang tan tlangpui. Tin, heng symptoms nasat dan hi mi hrang hrangah a inang lo thei bawk. Simptom langsar tak tak hmuh theih te hi han thlir ila.
| Taksa peng/system | Hmuh theih thil chi hrang hrang |
|---|
| Lu leh hmai | Vun hrual, mit inkar zau tak, leiah hian grooves thuk tak, beng, hmui leh hmui pangngai aia lian. |
| Nails a ni | A tlangpui aia thick zawk. |
| Vun | Vun a vawt. Vun hmun thenkhat (a bik takin folds, armpits leh neck-ah) a thim leh a thim thin. Hei hi damdawi lam chuan acanthosis nigricans tia hriat a ni. Heng hmunte hi khawih chuan velvety angin a lang thei. |
| Ha | A tlangpui aia lian, mipui inzawmkhawm, emaw, a hun hmaa ha chhuah. |
| Chhungril taksa peng hrang hrang | Kidney, heart, leh sexual organs (mipaah penis, hmeichhiaah vagina) chu a pangngai aia lian a ni. |
| Thil awm dan tlangpui dangte | Taksa sam sei lutuk, nau piang hma leh pian hnua sei zawi, pum a hring, vun hnuaia thau tlem lutuk, leh taksa ruh chak lo. |
Hemi avanga natna dang lo awm thei te
Heng basic symptoms bakah hian he syndrome hian natna khirh tak dang a thlen thei bawk.
- Hmeichhe naupang ovary- a cyst awm.
- Zunthlum . Hei hian a châng chuan nunna atana hlauhawm tak, ketoacidosis , a thlen thei a ni.
- Kidney lama harsatna nei.
Engtin nge diagnosis hi siam a nih?
He natna hi hriatchhuahna atana harsatna pakhat chu natna dang, a inang (Donahue syndrome ang chi) atanga thliar hran hi a ni. I fa
doctor chuan taksa chu uluk takin a enfiah ang a, i fa natna lan chhuah dan leh chhungkaw hriselna chanchin a zawt ang a, i fa
thisen sugar leh insulin level enfiah nan thisen test vawi engemaw zat a ti mai thei bawk Hei chauh hi diagnosis dik tak siam theihna kawng awmchhun a ni.
Engtin nge enkawl a nih?
Rabson-Mendenhall syndrome enkawlna atan hian team lian tak, specialist hrang hrang, surgeon leh dentist hrang hrangte puihna a ngai tlangpui. Chhungkaw tan pawh he natna hlauhawm tak nei fa neih avanga lungkhamna leh rilru natna lo awm thinte enkawl nan counseling hi a pawimawh hle thei bawk.
Tunah hian he natna hi damdawi nghet tak a la awm lo. Enkawlna hian natna lan chhuah dan control leh enkawl a tum a ni.
Enkawlna hi symptom tinrengah a bik takin a awm fo thin. Entirnan, Ovarian cyst paih chhuahna tur surgery, ha lama harsatna nei tan ha enkawlna, etc. A then phei chuan doctor-te chuan insulin hman tichaktu damdawi dang emaw, damdawi dang emaw dose sang tak an pe thin a, mahse hengte hi hun rei tak chhung atan chuan a hlawhtling lo fo thin.
Enkawlna thar chhui mek a ni
Mithiamte chuan insulin resistance na tak nena inzawm thisen sugar level sang (hyperglycemia) enkawlna kawng tha zawk an zirchiang chhunzawm zel a ni. Heng enkawlnate hian rah duhawm tak tak nei mahse, zirchianna neih belh a ngai a ni.
- Biguanides: Heng damdawi chi khat a ni a, taksain sugar a siam chhuah a tihtlem bakah insulin hman a tipung bawk .
- Leptin: He protein hian thisen sugar leh insulin level control nan a pui tih hmuhchhuah a ni.
- rhIGF-I (Recombinant insulin-like growth factor I): He protein hi ketoacidosis enkawlna atan hman a ni a, hei hi insulin resistance nasa tak avanga lo awm a ni .
Take-Home Thuchah a ni
- Rabson-Mendenhall Syndrome hi genetic condition a awm lo hle a, taksain insulin a hmang dik thei lo.
- Hei hi naupangin nu leh pa pahnih atanga a rochun gene chhia (`INSR` gene) avanga lo awm a ni.
- Naupan lai atanga a lan chhuah dan hian taksa thanna , hmel lan dan , vun, ha, leh chhungril lam taksa peng hrang hrang a nghawng a ni.
- Hei hi damdawi nghet tak a awm lo va, enkawlna hian a natna lan chhuah dan enkawl a tum a ni. Hei hian specialist doctor team te puihna a ngai a ni.
- I fa hian heng symptoms zinga pakhat tal a lantir a nih chuan doctor (doctor) thiam tak hnenah thurawn dil nghal a pawimawh hle.
Rabson-Mendenhall Syndrome, insulin resistance, genetic natna, naupang natna, acanthosis nigricans, ketoacidosis, zunthlum
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